After
34 years or so of trying to find answers to my disease and its
complications, you would think I am done searching. But, alas, that
is not the case.
My
newest dilemmas (yes, there are several):
My
liver enzymes are on the edge: if I add any medication to my current
cocktail, they shoot up. Is this something to worry and watch?! (it
is for me, at least)
I
need to exercise more, but I have symptoms that prevent me from
doing much: shortness of breath, chest tightness, dizziness. Are
these from the heart? Or is the source of this discomfort elsewhere?
Can it be removed and how?
My
blood pressure is still odd (systolic too high, diastolic too low).
Should it be medicated?
My
total cholesterol is 184mg/dl, the lowest it's ever been, but my LDL
is still high, at 145. Should we try some other medication? Or
should we say this is 'enough' for me, since my 'natural' numbers
are in the 500's and stay on the regimen and the diet I am on now ?!
So,
with these questions in mind, I proceeded to see my cardiologist and
my new lipid specialist. I got some answers, but as it is usually the
case with me, I don't agree with some of them. Or at least, I want to
learn more before I blindly follow their advice.
As
a reminder, this blog offers no advice to anyone. It is just a
representation of my struggles, my fears, and the choices I make
alone. It should never be read or interpreted otherwise.
The
Liver Levels
Following
up from the last entry
(http://livingwithfh.blogspot.com/2017/12/a-big-move-new-start-managing-same.html),
I have had my liver levels redone, because at the time I was writing
that entry they were elevated. What had gone up before were my
transaminases (which, I learned in the meantime, are a different
measurement of liver health than your bilirubin – more in a sec).
My
ALT
was 102 (normal up to 54 U/L) and my AST is 54 (normal up to 41 U/L).
At that time, my cardiologist believed that they were elevated
because on top of my usual cocktail I had taken about two weeks of
antibiotics for a UTI. After a week from stopping the antibiotics and
repeating the tests, the ALT is 59 (still a bit high) and the AST is
normal, at 34.
As
a reminder, my usual cocktail includes Lipitor, Zetia, Praluent, and
Warfarin (amongst other things), all of which are known to affect the
liver.
The
cardiologist is not concerned
at all
that the medicine I am taking is affecting the liver, although she
agrees that it seems that if we add anything else to it (like the
antibiotic) the levels rise. However, she does not consider these
values too elevated.
When
I personally see numbers jump around past the accepted range
(especially double, like the ALT), it makes me wonder whether
something is
wrong. I have not found out where to find the real answer, really.
For right now, I just know that my liver is sensitive to adding more
'poison' to it: after reading a whole bunch of things about what
really makes the transaminases go up, I am more careful with and
aware about the drugs or foods that do affect the liver (the warning
is usually printed on the labels) that I consider taking. So, I know
if I take Tylenol, it could affect me; if I drink a glass of alcohol,
it could also affect me, or the liver, rather.
This
is yet another thing to watch, I believe, because in this case the
liver damage is irreversible and potentially fatal. So, onward we go,
checking the liver enzymes every 3-4 months, in line with when we
check the cholesterol values. This is a decision I made despite my
cardiologist saying that these could be checked once every year now.
I just would rather not go off my life-long schedule of every 3-4
months which gives me reassurance that I know how my liver is doing
at all times, given the tendency of these numbers to spike. As you
know from reading here: I like to know more rather than less.
My
Heart Discomfort and the Need to Exercise More
I
had a little bit of a spat with my cardiologist (who is new to me,
having just moved to this part of the country). We have been over my
family history; she knows about my HoFH (which she stubbornly calls
'hyperlipidemia' – a term I am not crazy about because it is not
specific enough.) She also knows about my heart surgery and its
complex nature. She has not, however, seen any heart images yet, nor
has she asked me what my diet was. She declared that my 'heart is
just great' and suggested for a therapy going forward that I 'should
look at my diet and exercise'. I was a bit floored, and I felt like I
was not being heard.
This
is one of the peeves I have with just about every other doctor that
sees me: their failure to understand that just diet and exercise
alone will not make a difference in my case. Plus, she has no true
understanding of what my heart really is
doing:
yes, she has seen the transcripts from my previous echos and cath,
but she has not done one recently herself.
She
had also not asked me if I have any limitations in exercising – a
thing which I brought up myself. She was puzzled as to why. I then
shared that I am short of breath, and that I don't last very long as
I walk on an incline, I have the dizzy spells, and tinnitus quite
frequently. Should those be looked into?! I also asked her if the
Aortic Insufficiency and the weird blood pressure could be things
we're missing about my heart. I wished she should have found these,
and she should have suggested that we should look more into them on
her own, but I am happy she eventually listened to my list of
concerns. By now, I am used to helping
my doctors along when I see that they gloss over my case as just
another 'lazy patient who needs to cut McDonald's out of her diet'
(which I am far from, as you know).
I
hate arguing with doctors. I really do. I do expect them, though, to
step out of the text book and look at the patient as a unique human
being, with unique characteristics and responses to medicine, as well
as with a unique build which might be more or less responsive to
whatever the 'book' tells them to prescribe.
After
pushing for answers she agreed that she should hook me up to a holter
monitor for 48 hours and also do a cardio-pulmonary stress test, just
to understand more about my heart function during a normal day as
well as during exercise. So, I hope we get a better picture of what
this 'great heart' is really up to, so we can hopefully start on the
right foot.
In
the meantime, I agreed to push myself a little more each day, in my
walks: go a little further, walk a little faster, and see if my heart
takes it (although if history serves right, I tried this many times
before, and there is always this big wall I hit). We moved down from
The Rockies into the hills of North Carolina because my heart does
feel better here. So, maybe, just maybe, my insufficiency, caused by
who knows what, is less prohibitive of my movements here. I do know
that the heart muscle is happiest and healthiest when it moves. So,
I'll try more, if I can.
The
'Weird' Blood Pressure
My
new cardiologist suggested kind of nonchalantly and very
unconvincingly that I 'should be on an Ace-inhibitor, like
Lisinopril'. I asked her why, and she said it would lower my blood
pressure, but it 'is indicated that people with heart disease should
be on one, just as a safe measure.' The trouble with this is that my
blood pressure is not consistently high. It has higher spikes, and my
diastolic value is always too low. Every time I took something to
lower it, it made me dizzier than ever and I felt like fainting. So,
we decided to continue to monitor the numbers and see what the
consistent trend is: the high systolic (rare) or the normal systolic
and the low diastolic (more often than not)?! So, just like before,
we are just watching the blood pressure with no remedy. She thinks,
as the cardiologist before her, that my Aortic Insufficiency is to
blame for my odd blood pressure, but there is nothing to be done for
that.
My
LDL Level. Can It Be Lowered Even More?!
As
I mentioned in the last post, this cardiologist will not be the one
managing my lipids and their treatment. For that, she referred me to
an endocrinologist who specializes in lipids.
Incidentally,
I had met with this same endocrinologist almost 20 years ago when I
first came to this country. He happens to be renowned for his work
with lipids, so my PCP back then sent me to him as a 'know all'
specialist who will figure our my cholesterol problem.
Back
then, I had not been positively diagnosed with HoFH and I cannot
remember what he guessed on my disease: he did agree I had FH, but I
cannot remember if he picked a type. 20 years ago, his first move for
my treatment was to cut my Lipitor dose way down and to prescribe
Niacin (and later Niaspan) to me in addition to Lipitor. He is
probably the biggest believer in Niacin out of any doctor I have ever
seen. Niacin is, besides awful to take because of severe side
effects, ineffective to me: my numbers do not change on Niacin.
Now,
20 years later, guess what?! He recommends cutting the Lipitor in
half, and he once again
prescribes adding Niacin to
my drug regimen.
I politely told him we have been down that
path and that didn't work so well for me. So, I told him it's not
even worth talking about it, I will just not do it. He said that the
numbers are not so much of importance, but that the effects Niacin
has on the artery wall, which are proven to be significant, are much
more important. This is the second doctor that says this to me, which
puzzles me so: why do we have numbers and why do we strive for a
target if they are not
important?!
Using
the same logic, we could
say that an LDL of 145 mg/dl is indeed good enough and we should stay
where we are. Especially since at the last carotid ultrasound it was
seen that there was no more additional damage done within the past
year to my carotids. So, the artery wall is fine, why not stop here?!
He
mentioned I could now be on a more advanced form of Niacin, called
Enduracin which has less side effects. He asked me to consider this.
I am planning to get educated about Enduracin, but … I am not sure
what to think. So far, it looks like Enduracin is just a fancy name
for Niacin: I am not coming up with anything different for it than
what I have known about Niacin.
In
addition to Niacin and cutting the Lipitor in half, he also wants to
add Juxtapid (Lomitapide) (https://en.wikipedia.org/wiki/Lomitapide)
to my 'cocktail'. This is an orphan drug approved only for HoFH and
it is very potent. What it does is block fat from coming out of the
liver. From what I have read, it is very 'poisonous' to the liver,
potentially causing fatty liver disease, and high transaminases
levels – and again, we come full circle: I must watch those, as
well, so anything with known
side effects of liver damage scares me.
He
agreed that this is an incredibly potent medication which could
damage the liver, the reason for which he will prescribe a low dose
(5 mg) and he will cut my Lipitor dose from 80 to 40 mg. I told him I
must read and document myself more about this drug, before he can
start the proceedings with my health insurance to get me approved for
it. By all means, if you are reading here and have some input on this
drug, a personal experience with it, what the results were, etc,
please do share.
He
was not too concerned with the liver enzymes already being too high
for me. This is his opinion on those:
Transaminases
are not 'too high' unless they are consistently triple
the upper acceptable value, which mine have not been (yet).
And
He
believes that elevated transaminases (ALT and AST) are not
a good indicator of liver damage. That 'unless the bilirubin is
elevated, then there is no liver damage.' And my bilirubin has been
normal historically.
Again,
deep down in my heart, I believe there are numbers for a reason and
when they are not within guidelines they cause reason for concern. I
am not used to just ignore numbers. It's just not how I operate, no
matter how many specialists I am exposed to. I am always skeptical of
just ignoring the levels for any test!
If
I start taking Juxtapid, to prevent the liver from overloading with
fat and to prevent stomach upset and other GI issues, I will not be
able to eat any
fats at all.
My diet must be 100% fat free. I am not sure that this is totally
humanly possible for anyone. For instance, the plant based spread I
use sparingly as a butter substitute, or nuts, or the fish I eat a
couple of times a week, or the little bit of olive oil in my cooking
must be completely cut out. At that point, I think eating out would
be completely prohibited, too, as we have no control over what is in
those foods.
He
also said that even before taking Juxtapid, adding psyllium (or
Metamucil) to my diet will also remove any fat or cholesterol I am
eating now, even in small quantities and it will lower the numbers by
10-15%. The percentage is small, but I will take it. Because my liver
is so critical to me (if you have not gathered that by now!), I am
willing to try things that are known not to damage it. I have way too
many problems already to add liver failure to them, I think.
I
also asked about whether it would be important to the raise my HDL
which, for me, has always been low. It is 33 mg/dl now. He admitted
that he does not know how to improve that. He said beyond a glass of
wine and nuts daily, there is nothing medical that can improve the
HDL.
Right
now, I am in a 'research and wait phase': I am researching Juxtapid
and Enduracin, and I am waiting the results from my cardiac tests
(the holter monitor and the stress test). I am nervous about removing
half of the dose of Lipitor from my regimen, because Lipitor has
helped so much and I have gotten where I can tolerate the side
effects from it now. Removing it, playing with the dose and
introducing a drug that we're not sure it would work, or that it
would be safe in the long run makes me very nervous.
And
then, there is the Niacin. I really think that's an old school
remedy, with little success for FH people, but … should I give it
another go hoping that the drug has been improved over the years?!
And that's just the thing: is it a drug or more of a supplement which
is very little for a disease like FH which seems to be affected only
by powerful drugs, not supplements and diets? With everything I am
taking together, adding anything or taking anything away changes the
balance not only in treating the cholesterol, but also in the way I
feel and in the way I can (or cannot) manage all the side effects.
There is also the Warfarin to keep in mind which is affected by
anything
new, and which is worse: it is all processed in the liver.
And
then there is the nagging question: Are my numbers really the lowest
they can ever be and we should just not bother the magical mix of
drugs and diet we have found to achieve them?! Or is there such a
thing as trying more/ harder drugs to get those numbers evern lower?!
I wish it could be a clear-cut answer. But all of us who have
navigated this ship before know that it's mostly a guessing game.
Happy
Holidays to all, and I wish you all good health and easy decisions in
the New Year!
